Shujiro Hayashi

UVA IRRADIATION FOLLOWING TREATMENT WITH TOPICAL 8-METHOXYPSORALEN IMPROVES BLEOMYCIN-INDUCED SCLERODERMA IN A MOUSE MODEL, BY REDUCING THE COLLAGEN CONTENT AND COLLAGEN GENE EXPRESSION LEVELS IN THE SKIN

BACKGROUND Recent studies have demonstrated that systemic or topical PUVA therapy, i.e., ultraviolet A (UVA) irradiation following treatment with 8-methoxypsoralen (8-MOP), is effective against the sclerotic skin lesions in systemic sclerosis. However, the mechanisms still remain unknown. OBJECTIVE To clarify the mechanisms of this therapy, we created a mouse model of bleomycin (BLM) injection-induced scleroderma and evaluated the effects of PUVA on the fibrotic lesions of scleroderma in this mouse model. METHODS BLM was injected subcutaneously once a day into the mice for 24 days. During the injection period, one group of mice was irradiated with UVA following local application of 8-MOP. Control groups were also set up, which were injected with phosphate-buffered saline, instead of BLM. Skin tissue samples examined histopathologically changes, measured of the content of hydroxyproline, and checked for the expression of genes encoding type I collagen, type III collagen, and transforming growth factor-β1 (TGF-β1). RESULTS The mouse models of scleroderma was found to show an increase in the density of the collagen fibers and thickening of the dermis and increased expressions of type I collagen, type III collagen, and TGF-β1. However, the combination of BLM treatment and topical PUVA treatment mice appeared reduced the dermal thickness and hydroxyproline content, down-regulation of expressions of the type I and type III collagen genes was observed while the expression of the TGF-β1 gene remained unchanged. CONCLUSION These results suggest that the effectiveness of topical PUVA therapy is attributable to the down-regulation of the expressions of the collagen genes by this treatment. The results additionally suggest that is not mediated by down-regulated expression of the TGF-β1.

Intramedullary and Retroperitoneal Melanocytic Tumor Associated With Congenital Blue Nevus and Nevus Flammeus: An Uncommon Combination of Neurocutaneous Melanosis and Phacomatosis Pigmentovascularis—Case Report

Neurocutaneous melanosis (NCM) is a rare condition characterized by central nervous system melanocytic tumors associated with congenital melanocytic nevi. Phacomatosis pigmentovascularis (PPV) is an association of vascular nevus with pigmentary nevus. Aberrant maturation of neural crest-derived cells is considered to be related to pathogenesis in both conditions. However, association of NCM and PPV has not been reported to the best of our knowledge. Melanocytoma, which usually involves the leptomeninges or spinal cord, is extremely rare in the retroperitoneum. We present here a case of a patient with NCM, PPV, and melanocytic tumors in the spinal cord and retroperitoneum, which were treated surgically. A 40-year-old woman had a 2-year history of dysesthesia and weakness in the left leg. History included congenital giant blue nevus-like lesion in the trunk, a port-wine stain in the sacral area, and Caesarean section performed 8 years before, when diffuse pigmentation in the peritoneum was noted. Magnetic resonance (MR) imaging of the spine revealed an intramedullary tumor at T10 level with paramagnetic signal characteristics. The spinal cord tumor was totally removed, and the histological diagnosis was melanocytoma. Three months later, a left retroperitoneal mass with histological features of melanocytic tumor was removed. Neither tumors recurred and the patient stays ambulatory 4 years after the surgery. Multiple subtypes of melanocytic tumors with distinctive features of NCM and PPV can develop simultaneously, mimicking malignant melanoma. Gross total resection of each tumor, when indicated, is beneficial.

Fixed drug eruption caused by noscapine

Dear Editor, A fixed drug eruption characteristically recurs at the same site or sites each time the offending drug is administrated; however, with each exposure, the number of involved sites may increase. The number of drugs capable of producing fixed drug eruptions is very large; however, most fixed drug eruptions are caused by a limited number of substances. An earlier series incriminated particularly analgesics, sulphonamides and tetracyclines. In a report from Finland, phenazones were reported to be the cause of most eruptions, with barbiturates, sulphonamides, tetracyclines and carbamazepine causing reactions in a smaller number of patients. A series from India implicated acetylsalicylic acid as the most common cause of fixed drug eruptions in children. Many of the antitussive agent-induced fixed drug eruptions are caused by tipepidine hibenzate. Noscapine is a centrally-acting non-opioid antitussive agent and is contained in many over-the-counter (OTC) cold medications. However, only one case of fixed drug eruption caused by noscapine has been reported to date. We report a case of fixed drug eruption with unique clinical features caused by noscapine. A 57-year-old man visited our department on 16 October 2007 complaining of a pruritic eruption on the right lower leg. Past medical and family histories were unremarkable. The patient reported taking OTC drugs whenever he suffered from the symptoms of common cold; he was currently on no medication. He had first noticed a pruritic erythematous lesion measuring approximately 50 mm in diameter on the inner aspect of the right lower leg approximately 1 year prior, and the lesion had gradually increased in size. Physical examination at presentation revealed an elliptical erythematous lesion, 140 mm · 110 mm in size, on the inner aspect of the right lower leg (Fig. 1a). The margin of the erythema showed pigmentation with scales, and the center of the erythema

Patient with extensive Mongolian spots, nevus flammeus and nevus vascularis mixtus: A novel case of phacomatosis pigmentovascularis.

ketoprofen suggested his photosensitivity was induced by cross-sensitization to photoproducts of fenofibrate and ketoprofen. It became well known that ketoprofen is a causative agent of photosensitization since it was marketed. Recently, cross-photosensitization between ketoprofen and related NSAIDs, such as tiaprofenic acid or suprofen, has proved by photopatch test. Tiaprofenic acid and suprofen both have a similar structure to ketoprofen. Meanwhile, ketoprofen and fenofibrate both have a benzophenone structure (Fig. 1b). Cross-photosensitization between fenofibrate and ketoprofen has likewise demonstrated by photopatch test. The action spectrum of ketoprofen photosensitivity is in UVA, whereas that of fenofibrate has been reported in UVA or UVB or both. In our case, photopatch tests of fenofibrate and ketoprofen were positive in both UVA and UVB. In Japan, although the drug information of ketoprofen has warned this cross-reactivity, there is no description in that of fenofibrate. Thus, we have submitted a drug safety information report to the Ministry of Health, Labour and Welfare, Japan. We should be aware that fenofibrate can cause photosensitivity in patients whom it has been treated with ketoprofen before. CONFLICT OF INTEREST: None declared.

Patient with the vascular type of Ehlers–Danlos syndrome, with a novel point-mutation in the COL3A1 gene

reported fact that there is no correlation between clinical severity and emotional QOL in female AA. The PIADS and VAS scores of the responders (RS 1) and non-responders (RS 0) were not significantly different by Mann–Whitney’s Utest and the PIADS and VAS scores did not correlate with the RS by Spearman’s rank correlation coefficient. However, this may be ascribed to insensitivity of the RS. Overall, mean standard deviation of the VAS was 7.91 1.74, indicating patients’ sufficient satisfaction with wigs. According to the recent British guidelines for AA, a wig or hairpiece is recommended as a good practice point but its evidence is only based on expert opinion or consensus. Here, we quantitatively and statistically examined whether wigs or hairpieces are useful by using PIADS and found that wigs or hairpieces improve perceived competence, adaptability and self-esteem, and the effect correlates with the extent of satisfaction with appearance using wigs.

Tracing the origins of active amide group-positive drug-induced pemphigus vulgaris along the Silk Road: a case report of candesartan-induced pemphigus vulgaris and review of nonthiol drug-induced pemphigus

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Palmoplantar pustulosis and pustulotic arthro-osteitis treatment with potassium iodide and tetracycline, a novel remedy with an old drug: a review of 25 patients

The use of potassium iodide (KI) to treat palmoplantar pustulosis (PPP) and pustulotic arthro‐osteitis (PAO) has not previously been reported. Here, we report the first successful treatment of PPP and PAO with KI.